AB1295 TREATMENT OUTCOMES OF ADULT-ONSET STILL’S DISEASE PATIENTS WITH BIOLOGICAL THERAPY
Authors: Y. Erez, T. Demirci Yildirim, M. Birlik, G. Can, F. Onen, İ. Sari
Journal: Annals of the Rheumatic Diseases (2022)

Background Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder with heterogeneous distrubition (1). Due to rarity of AOSD and limited controlled study about efficacy of biologic treatment in AOSD, management of refractory AOSD patients poses several challenges. Objectives The aim of this study to investigate clinical characteristics and treatment outcomes of AOSD patients who receive biological therapy. Methods Patients were identified who met Yamaguchi classification criteria between January 2010 and December 2021 from our database. AOSD patients treated with biologic treatment were included in this study. Demographic data, clinical features and treatment characteristics were recorded. Results 49 AOSD patients were identified from database. 9 AOSD patients with biologic therapy (7 female, mean age 42.3±19) were included in this study. Mean age at diagnosis was 31.5± 16.7 and mean follow-up period was 9.3± 5.5 years. Median drug retention duration was found 95 (5-150) months. It was established that all patients used high dose steroid at diagnosis and 3 (33.3%) out of them received pulse steroid therapy. Initial therapy was methotrexate for all patients and leflunomid was used secondly as monotherapy or combination therapy after methotrexate inefficacy. It was observed that indication for biologic therapy was arthritis and systemic inflammatuar symptoms in 5 patients. 4 patients was initiated biologic therapy for refractory arthritis without systemic inflammation findings. First biologic agents were tocilizumab and etanercept in 3 patients, infliximab and anakinra in 2 patients. It was shown that biological therapy was switched in 3 patients due to inefficacy and 2 patients because of side effects. Allergic reaction developed in 2 patients treated with infliximab and infliximab switched to adalimumab and etanercept. Biological therapy was discontinued in 2 patients due to remission, and patients remained in remission after cessation of biological agent. None of patients had serious infection. Hemophagocytic syndrome was developed in only one patient during disease course. Conclusion According to our cohort, one of five AOSD patients received biological therapy during follow-up. Our results revealed that the need for biologic therapy during the course of the disease is not uncommon in AOSD, and that biologic therapy is essential for maintaining remission in resistant patients. References [1]Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still’s disease. Semin Arthritis Rheum. 2006 Dec;36(3):144-52. doi: 10.1016/j.semarthrit.2006.07.001. Epub 2006 Sep 1. PMID: 16949136. Table 1. Baseline clinical and treatment characteristics (n=9 ) Female, n (% ) 7 (77.7) Age, years 42.3±19 Age at diagnosis, years 31.5± 16.7 Smokers, n (% ) 6 (66.6) Drug retantion duration, months 95 (5-150) Follow-up time, years 9.3± 5.5 Treatment characteristic n (%) Biologic therapy indication -Systemic inflammation findings 5 (55.5) -Refractory arthritis 4 (44.4) İnitial biologic therapy Etanercept 3 (33.3) Tocilizumab 3 (33.3) Infliximab 2 (22.2) Anakinra 2 (22.2) Switching agent 5 (55.5) Cessation of biologic therapy 3 (33.3) Disclosure of Interests None declared